mortality/aging
• mice are born at expected Mendelian ratios; however, all neonates die within hours after birth
|
homeostasis/metabolism
respiratory system
• at P0, the nasal capsule is missing
|
• at E17.5, the nasal septum cartilage is abnormally ossified and malformed
|
• thyroid cartilage is abnormally ossified at P0
|
behavior/neurological
• newborns are unable to suckle
|
craniofacial
• at P0, all mice exhibit severe craniofacial malformations
• however, embryos are grossly normal at E10.5 and E12.5
|
• newborns show severe craniofacial skeleton defects due to accelerated osteochondrogenic differentiation; the NCC-derived nasal capsule, maxilla, premaxilla, mandible, tympanic ring and body of hyoid bone are absent or severely malformed
• in contrast, mesoderm-derived skeleton elements including the parietal, lateral portion of the interparietal, supraoccipital, exoccipital, basioccipital and otic capsule are formed normally
|
• hyoid bone is heavily ossified at P0
|
• newborns exhibit an over-ossified body of the hyoid bone
• premature ossification in the body of hyoid bone is first seen at E15.5
|
• incisor tip is abnormally ossified at E14.5 and E15.5
|
• newborns exhibit a hypoplastic and malformed mandible
• mandibular bone is heavily ossified at P0
|
• at P0, the angular process is missing
|
• at P0, the condylar process is missing
|
• at P0, the coronoid process is missing
|
• at E16.5, E18.5 and P0, the mandible is severely shortened
|
• newborns exhibit a hypoplastic and malformed maxilla
|
• newborns exhibit a hypoplastic and malformed premaxilla
|
• at E16.5, E18.5 and P0, the maxilla is severely shortened
|
• at P0, mice exhibit a domed skull
|
• neonatal palatal bones are severely hypoplastic and thus palate is cleft
|
• at P0, the nasal capsule is missing
|
• palatal shelves are well developed and elevate to the horizontal position above tongue but fail to elongate or fuse at E14.5
• however, in vitro, palatal shelves show complete fusion with normal disappearance of the medial edge epithelium after 72 hours in organ culture
|
round face
(
J:254756
)
• at E14.5, embryos exhibit a round face
|
• newborns exhibit a complete cleft palate caused by craniofacial skeleton defects
|
• at E16.5 and E18.5, the tongue is arched rather than flat
|
• at E16.5, E18.5 and P0, mice exhibit an exposed (protuberant) tongue
|
• at E17.5, the nasal septum cartilage is abnormally ossified and malformed
|
short snout
(
J:254756
)
• at E14.5, embryos exhibit a short snout
|
skeleton
• newborns show severe craniofacial skeleton defects due to accelerated osteochondrogenic differentiation; the NCC-derived nasal capsule, maxilla, premaxilla, mandible, tympanic ring and body of hyoid bone are absent or severely malformed
• in contrast, mesoderm-derived skeleton elements including the parietal, lateral portion of the interparietal, supraoccipital, exoccipital, basioccipital and otic capsule are formed normally
|
• hyoid bone is heavily ossified at P0
|
• newborns exhibit an over-ossified body of the hyoid bone
• premature ossification in the body of hyoid bone is first seen at E15.5
|
• incisor tip is abnormally ossified at E14.5 and E15.5
|
• newborns exhibit a hypoplastic and malformed mandible
• mandibular bone is heavily ossified at P0
|
• at P0, the angular process is missing
|
• at P0, the condylar process is missing
|
• at P0, the coronoid process is missing
|
• at E16.5, E18.5 and P0, the mandible is severely shortened
|
• newborns exhibit a hypoplastic and malformed maxilla
|
• newborns exhibit a hypoplastic and malformed premaxilla
|
• at E16.5, E18.5 and P0, the maxilla is severely shortened
|
• at P0, mice exhibit a domed skull
|
• neonatal palatal bones are severely hypoplastic and thus palate is cleft
|
• at P0, the nasal capsule is missing
|
• at E17.5, the nasal septum cartilage is abnormally ossified and malformed
|
• thyroid cartilage is abnormally ossified at P0
|
• mice exhibit abnormal ossification within the maxilla and mandible, nasal septum, hyoid and laryngeal cartilages
|
• premature ossification in the body of hyoid bone is first seen at E15.5
|
• newborns exhibit premature frontal suture closure (craniosynostosis)
|
vision/eye
• at E14.5, embryos exhibit hypertelorism
|
hearing/vestibular/ear
• neonatal tympanic rings are thickened and deformed
|
nervous system
N |
• morphology of cranial nerves is grossly normal at E10.5 and E12.5
|
• size of sympathetic ganglia is reduced at E16.5 and E17.5
|
• size of dorsal root ganglia is reduced at E16.5 and E17.5
|
digestive/alimentary system
• neonatal palatal bones are severely hypoplastic and thus palate is cleft
|
• palatal shelves are well developed and elevate to the horizontal position above tongue but fail to elongate or fuse at E14.5
• however, in vitro, palatal shelves show complete fusion with normal disappearance of the medial edge epithelium after 72 hours in organ culture
|
• newborns exhibit a complete cleft palate caused by craniofacial skeleton defects
|
• at E16.5 and E18.5, the tongue is arched rather than flat
|
• at E16.5, E18.5 and P0, mice exhibit an exposed (protuberant) tongue
|
growth/size/body
• incisor tip is abnormally ossified at E14.5 and E15.5
|
• neonatal palatal bones are severely hypoplastic and thus palate is cleft
|
• at P0, the nasal capsule is missing
|
• palatal shelves are well developed and elevate to the horizontal position above tongue but fail to elongate or fuse at E14.5
• however, in vitro, palatal shelves show complete fusion with normal disappearance of the medial edge epithelium after 72 hours in organ culture
|
round face
(
J:254756
)
• at E14.5, embryos exhibit a round face
|
• newborns exhibit a complete cleft palate caused by craniofacial skeleton defects
|
• at E16.5 and E18.5, the tongue is arched rather than flat
|
• at E16.5, E18.5 and P0, mice exhibit an exposed (protuberant) tongue
|
• at E17.5, the nasal septum cartilage is abnormally ossified and malformed
|
short snout
(
J:254756
)
• at E14.5, embryos exhibit a short snout
|
cardiovascular system
N |
• morphology of the cardiac outflow tract and cardiac development is grossly normal at E17.5
|
embryo
N |
• both cranial neural crest cell (CNCC) migration and CNCC proliferation are normal
|